甲状旁腺癌（PC）是一种罕见的内分泌恶性肿瘤，也是原发性甲状旁腺功能亢进症的罕见原因。转移性疾病预后谨慎，可用的全身治疗选择有限。我们描述了一名 64 岁女性继发于 PC 的原发性甲状旁腺功能亢进症的病例。尽管最初进行了手术切除，但患者在 6 个月内复发，出现广泛的脑和骨骼转移性疾病。她出现了日益恶化的甲状旁腺激素介导的高钙血症，这种情况对增加剂量的西那卡塞和抗吸收治疗无效。分子基因组学鉴定出恶性组织内的高肿瘤突变负荷，并进行了单药纳武单抗免疫治疗。服用一剂后，难治性高钙血症和原发性甲状旁腺功能亢进症得到缓解。患者已耐受持续 3 周周期的纳武单抗治疗。截至 2024 年 6 月，她仍处于生化缓解状态，即开始纳武单抗治疗 12 个月后。© 作者 2024。由牛津大学出版社代表内分泌学会出版。

Parathyroid carcinoma (PC) is a rare endocrine malignancy and an uncommon cause of primary hyperparathyroidism. Metastatic disease confers a guarded prognosis with limited systemic treatment options available. We describe a case of a 64-year-old woman with primary hyperparathyroidism secondary to PC. Despite initial surgical resection, the patient relapsed within 6 months with widespread cerebral and skeletal metastatic disease. She developed worsening parathyroid hormone-mediated hypercalcemia that was refractory to escalating doses of cinacalcet and antiresorptive therapy. Molecular genomics identified high tumor mutation burden within the malignant tissue and single-agent nivolumab immunotherapy was administered. After one dose, there was resolution of her refractory hypercalcemia and primary hyperparathyroidism. The patient has tolerated ongoing treatment with 3 weekly cycles of nivolumab. She remains in biochemical remission as of June 2024, which is now 12 months after commencement of nivolumab.© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society.